Spontaneous ocular prolapse as an unusual late presentation of acrocephalosyndactyly.

Ophthalmic Plast Reconstr Surg

St. Paul's Eye Unit, Liverpool, United Kingdom.

Published: January 2008

A 38-year-old man presented with an unusual complaint of spontaneous left ocular prolapse, which had occurred while scratching his lower eyelid. He was noted to have an atypical facial appearance with frontal bossing and maxillary hypoplasia. Examination of the hands revealed bilateral syndactyly, suggesting a diagnosis of acrocephalosyndactyly. While it is common for eyes to prolapse in childhood in Crouzon or Apert syndrome, it is unusual for them to present in this way in middle age. It may be that age-related loss of tissue elasticity and consequent lower eyelid laxity decreased the inferior support of the globe, explaining the late presentation.

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http://dx.doi.org/10.1097/IOP.0b013e318142d162DOI Listing

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